Presentation of Supratentorial Ependymomas in Children

Symptoms

The symptoms and signs vary according to age group.

Infants

• Large tumors at time of diagnosis: Tumors in infants may be diagnosed late and with much larger sizes (sometimes hemispheric) than the signs and symptoms may suggest.
• Increased ICP symptoms: Symptoms tend to be nonspecific and related to elevated ICP, such as lethargy, vomiting, failure to thrive, delayed milestones, large head circumference, or bulging anterior fontanelle. 
• Neurologic deficits possible: There may be neurologic deficits such as weakness of an arm or leg, caused by the location of the tumor.

Young children

• Complaints referable to elevated ICP: Children who can talk may complain of headaches or blurred vision.
• Deterioration in walking: Often the gait will be unsteady or ataxic, or there may be diplopia from cranial nerve palsy.
• Seizures: Seizures can occur, but less commonly.

Older children and teenagers

• Increased ICP symptoms: The usual symptoms are of persistent headaches and blurred vision from increased ICP.
• Diplopia: Depending on the location of the tumor, there may be diplopia.
• Seizures: Seizures may sometimes occur.

Patterns of evolution

Two patterns are seen:

• Gradual is most common:  The onset of symptoms is subtle and slowly progressive. Infants may have nonspecific signs such as failure to thrive or poor feeding, while older children may develop mild weakness of an arm, clumsiness, or gait problems.
• Sudden, occasional: Acute hydrocephalus or hemorrhage may result in the child presenting in extremis with dilated pupils, seizures, or coma.

Time for evolution

• Gradual onset of symptoms may be noticed over several weeks before diagnosis.
• Sudden onset may be severe headache and decreased consciousness within hours.

Evaluation at Presentation

• See EVALUATION

Intervention

Depends on clinical presentation and level of consciousness.

Stabilization

For patients with a decreased level of consciousness the following steps should be taken:

• Evaluate: check vital signs – SaO2, pulse rate, blood pressure, respiratory rate, GCS score
• Start oxygen
• IV access
• Administer: Steroids and anticonvulsant medication.

Preparation for definitive intervention, nonemergent

• Steroids: Start oral steroids if neurologic deficits are present.
• Scan: Arrange for MRI of the brain.

Preparation for definitive intervention, emergent

• Administer:  Oxygen, IV steroids, mannitol (if pupils are unequal or dilated), and anticonvulsants.
• Scan: Arrange for urgent MRI or CT brain scan.
• Prepare for surgery: Notify pediatric anesthesiologist.

Admission Orders

• No special orders: Standard orders for a child with a brain tumor are used in the preoperative period.

CSF drainage parameters

If an EVD is inserted, the author’s preferences are to:

• Drain to low normal: CSF drainage chamber should be placed at ear level initially and adjusted according to pressure and volume of CSF draining out.
• Maintain low normal ICP: CSF volume drainage is monitored, and height of drainage chamber is adjusted to keep ICP in the range of 5–10 cm H2O.
• Maintain drainage: If ICP is continuously high, drain CSF continuously, with the height of chamber 5 cm above ear level.